Malignant primary cardiac tumors. The Cleveland Clinic experience, 1956 to 1986

Malignant primary cardiac tumors have uniformly been associated with poor long-term survival. We reviewed data on 11 patients with such tumors seen between 1956 and 1986. There were five men and six women, with a mean age at presentation of 44 years. Nine (82 percent) of 11 patients presented initially with respiratory symptoms, and 45 percent (five) presented with chest pain. No patient was asymptomatic on initial presentation. Forty-five percent (five) of the patients had elevated jugular venous pressure or a systolic murmur. Cardiomegaly was present in eight (73 percent) of 11 patients. Nine of ten patients had a hemoglobin level less than 12 g/100 ml, and 30 percent (3/10) presented with thrombocytosis. Seventy percent (eight) of the patients underwent surgical biopsy, and in 30 percent (three), surgical excision of the tumor was attempted. Angiosarcoma was the most frequent type of tumor (four patients), followed by malignant fibrous histiocytoma (three patients), mesothelioma (two patients), rhabdomyosarcoma (one patient), and primary lymphoma (one patient). Long-term follow-up data reveals that ten (91 percent) of 11 patients had died, with a mean duration from diagnosis until death of 9.7 months. Newer pathologic techniques for identification of sarcomas, aggressive surgical resection, and advanced chemotherapy for certain subgroups may contribute to better survival for patients with these tumors in the future. In addition, cardiac transplantation may play an important role in the future for those patients with localized disease.